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Year : 2018  |  Volume : 66  |  Issue : 11  |  Page : 1622

Corneal edema in xeroderma pigmentosa

Cornea and Anterior Segment Services, Tej Kohli Cornea Institute, L. V. Prasad Eye Institute, Hyderabad, Telangana, India

Date of Web Publication25-Oct-2018

Correspondence Address:
Dr. Sunita Chaurasia
Tej Kohli Cornea Institute, L. V. Prasad Eye Institute, L. V. Prasad Marg, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_469_18

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How to cite this article:
Chaurasia S. Corneal edema in xeroderma pigmentosa. Indian J Ophthalmol 2018;66:1622

How to cite this URL:
Chaurasia S. Corneal edema in xeroderma pigmentosa. Indian J Ophthalmol [serial online] 2018 [cited 2020 Nov 29];66:1622. Available from: https://www.ijo.in/text.asp?2018/66/11/1622/244061

Xeroderma pigmentosa is an autosomal recessive disorder characterized by a defect in the DNA repair mechanism because of which there is a damaging effect on the sun-exposed areas of the body.[1],[2] The various corneal changes in these patients include haze, scar, vascularization, edema, and stromal thinning.[3] Stromal edema on clinical examination is indicative of poor endothelial function in these eyes.[4],[5] A 25-year-old male with xeroderma pigmentosa presented with gradually decreasing vision in the right eye. The right eye revealed stromal haze and significant corneal edema distributed more in inferior cornea [Figure 1]a. The left eye had minimal stromal haze inferiorly [Figure 1]b. Descemet membrane endothelial keratoplasty was performed in the right eye [Figure 1]c. The visual acuity improved from 20/125 preoperatively to 20/25 postoperatively in the right eye at 1-month postoperative period. Specular microscopy of the left eye revealed a low endothelial cell density and increased mean cell area [Figure 1]d, as has been reported in an earlier study.[4]
Figure 1: (a) Slit lamp photograph of the right eye showing corneal edema and mild stromal haze. (b) Slit lamp photograph of the left eye showing scarring inferiorly. (c) Slit lamp photograph of the right eye 1 month after Descemet membrane endothelial keratoplasty. (d) Specular microscopy of the left eye showing reduced endothelial cell density and increased cell area

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This case supports that endothelial cell loss and corneal edema occurs in ceroderma pigmentosa and can be managed with Descemet membrane endothelial keratoplasty when seen at the stage of minimal stromal scarring.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kraemer KH, Lee MM, Scotto J. Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases. Arch Dermatol 1987;123:241-50.  Back to cited text no. 1
Goyal JL, Rao VA, Srinivasan R, Agrawal K. Oculocutaneous manifestations in xeroderma pigmentosa. Br J Ophthalmol 1994;78:295-7.  Back to cited text no. 2
Chaurasia S, Mulay K, Ramappa M, Sangwan V, Murthy S, Nair R, et al. Corneal changes in xeroderma pigmentosum: A clinicopathologic report. Am J Ophthalmol 2014;157:495-500.  Back to cited text no. 3
Mohamed A, Peguda R, Ramappa M, Ali MJ, Chaurasia S. Corneal endothelium in xeroderma pigmentosum: Clinical specular microscopy study. Br J Ophthalmol 2016;100:750-3.  Back to cited text no. 4
Chaurasia S, Ramappa M. Surgical intervention for corneal involvement in xeroderma pigmentosa. Int Ophthalmol 2012;32:1-2.  Back to cited text no. 5


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